Reporting and Description for Congenital Middle Ear Malformations to Facilitate Surgical Management

Yang, Feng; Yang, Liu

doi:10.1177/0003489418792939

Cited by 9 publications

(11 citation statements)

References 50 publications

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“…16,17 However, a few studies reported that the incus anomaly was predominant in their case series. [18][19][20] Other studies 5,21 have identified stapes and incudostapedial complex as the most commonly involved structure. In our study, isolated anomalies of the stapes and anomalies of the incus associated with the stapes contributed to the majority of the malformations found in surgery (29.0% and 50.3%, respectively).…”

Section: Discussionmentioning

confidence: 99%

Isolated Congenital Middle Ear Malformations: Comparison of Preoperative High-Resolution CT and Surgical Findings

Zhang

et al. 2019

Ann Otol Rhinol Laryngol

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Objectives: To compare preoperative temporal bone high-resolution computed tomography (HRCT) readings to intraoperative findings during exploratory tympanotomy for suspected cases of isolated congenital middle ear malformations (CMEMs) and summarize the malformations that can and cannot be diagnosed with HRCT. Methods: A retrospective study was conducted. All cases were confirmed as isolated CMEMs during surgery. Detailed clinical records were reviewed, with a focus on imaging and surgical findings. Results: One hundred and thirty-two patients and 145 ears were reviewed. Ninety cases (62.1%) could be identified as isolated CMEMs and at least one as middle ear anomaly using preoperative HRCT. Fifty-five cases (37.9%) were reported to be completely normal and the patients underwent exploratory tympanotomy to determine the final diagnosis. Stapes fixation, either alone or associated with other ossicular chain anomalies, contributed to 53.1% of the cases. Most cases of aplasia or dysplasia of the ossicular chain, for example, aplasia/dysplasia of the long process of the incus, aplasia of the stapes’ superstructure, and atresia of the oval window were easily identified in preoperative HRCT. However, fixation of the ossicular chain can be elusive in HRCT, and exploratory tympanotomy is needed for a definitive diagnosis. Conclusions: HRCT provides helpful preoperative clinical information in CMEM and may obviate the need for middle ear exploration in some cases. The negative findings (anomalies that are difficult to identify through preoperative HRCT) and the positive findings (anomalies that are relatively easy to identify through preoperative HRCT) were summarized.

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Section: Discussionmentioning

confidence: 99%

Isolated Congenital Middle Ear Malformations: Comparison of Preoperative High-Resolution CT and Surgical Findings

Zhang

et al. 2019

Ann Otol Rhinol Laryngol

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“…Yang [8] reviewed 41 articles with 662 cases published between 2000 and 2017 and reported that only 17 out of 189 cases with atresia of the oval window or a fixed stapes footplate demonstrated normal stapes superstructure. In a study of 97 ears with a missing oval window, no cases showed normal stapes, suggesting that the defects in the stapes were closely correlated with the absence of the oval window [9].…”

Section: Discussionmentioning

confidence: 99%

Two cases of congenital stapes malformation: Implications for development of the stapes footplate and the oval window

Ishida

Okano

Hayashi

et al. 2020

Acta Oto-Laryngologica Case Reports

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The origin of the stapes footplate has been controversial for decades, especially regarding involvement of the otic capsule in the development of the footplate. We report two cases of stapes malformation in which the stapes footplate was absent, while the oval window was formed but was covered with mucosal membrane. Both the patients visited our department for long-standing hearing loss, which was due to congenital ossicular anomalies of rare variation, as suggested by intraoperative findings. Through two operated cases, we have also discussed the origin and the mechanisms in the development of the stapes and the oval window. The findings suggest that the formation of the oval window is independent from the development of the stapes footplate. Further reports and accumulation of cases with ossicular malformation are required to develop an appropriate classification of congenital ossicular anomalies based on both developmental mechanisms and analyses of the surgical outcomes.

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“…12 Though more recently, it is suggested that type 3 anomalies occur most often. [13][14][15][20][21][22] In contrast to results in CMEA class 1 to 3 anomalies, surgery performed on class 4 anomalies has resulted in mixed long-term outcomes in several studies due to iatrogenic hearing loss and re-obliteration of the newly created window to the inner ear. 17,23,24 Also, in several studies it is suggested that middle ear surgery in patients with specific syndromes can be less effective to restore hearing than in non-syndromal patients.…”

Section: Introductionmentioning

confidence: 99%

Congenital Anomalies of the Ossicular Chain: Surgical and Audiological Outcomes

Henkemans

Smit

Stokroos

et al. 2021

Ann Otol Rhinol Laryngol

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Objectives: In this study, we aim to analyze audiometric outcomes of middle ear surgery in patients with congenital middle ear anomalies. Methods: In this single center retrospective cohort study, audiological outcomes were extracted from patient files. Patients with a congenital middle ear anomaly treated surgically in a tertiary referral center between June 2015 and December 2020 were included. Pre- and postoperative short- and long-term audiometric data (at ≥3 and ≥10 months respectively) were compared to analyze hearing outcomes. Results: Eighteen ears (15 patients) were treated surgically with an exploratory tympanotomy. At short term follow up statistically significant improvements in air conduction thresholds and air-bone gaps were found. Hearing improved in 94.4% (17/18) of operated ears. Successful outcome, defined as an air-bone gap closure to within 20 dB after surgery, was reached in 44.4% (8/18). Serviceable hearing (air conduction ≤30 dB) was reached in 55.6% (10/18). Negative outcome (any significant deterioration in hearing) occurred in 1 patient: in this ear otitis media occurred during the postoperative course. At long term follow up, available for 50% of the cohort, hearing remained stable in 5 ears, improved in 1 ear and deteriorated in 3, all of which underwent revision surgery. Sensorineural hearing loss due to surgery, or other complications, were not encountered. Conclusion: middle ear surgery was found to be an effective treatment option to improve hearing in this cohort of patients with congenital middle ear anomalies. Surgical goals of obtained gain in air conduction thresholds and serviceable hearing levels were met by most patients without the occurrence of any iatrogenic sensorineural hearing loss.

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Reporting and Description for Congenital Middle Ear Malformations to Facilitate Surgical Management

Abstract: The authors describe the different types of congenital middle ear malformations. This category description considers ear embryogenesis and is focused on stapes deformity. It may provide better understanding of disease development and guide modern hearing reconstructive surgery.

Cited by 9 publications

References 50 publications

Isolated Congenital Middle Ear Malformations: Comparison of Preoperative High-Resolution CT and Surgical Findings

Isolated Congenital Middle Ear Malformations: Comparison of Preoperative High-Resolution CT and Surgical Findings

Two cases of congenital stapes malformation: Implications for development of the stapes footplate and the oval window

Congenital Anomalies of the Ossicular Chain: Surgical and Audiological Outcomes

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